Honda Masataka
Department of Pediatric Nephrology, Tokyo Metropolitan Kiyose Children's Hospital, Tokyo, Japan.
Pediatr Int. 2002 Apr;44(2):210-6. doi: 10.1046/j.1328-8067.2001.01533.x.
The treatment of nephrotic syndrome (NS) has recently made dramatic progress. The ultimate purpose of treatment is that patients can lead a normal, disease-free life with no adverse effects from treatment. However, clear treatment guidelines remain to be established in the children with frequent relapsing NS (FRNS) and steroid-resistant NS (SRNS). The frequent use or large dose of steroids may lead to a serious adverse effect. Immunosuppressive drugs including cyclophosphamide and cyclosporin have been used to induce lasting remission, thereby sparing patients from further exposure to steroids. However, these drugs have both acute and chronic side-effects. Mizoribine is a relatively safe and effective immunosuppressant. The report discusses the role of mizoribine in FRNS and SRNS in children.
The papers in experimental and clinical studies about mizoribine in NS were reviewed. Our experiences of mizoribine were also added.
Mizoribine has been reported as an effective and safe drug for patients with FRNS. However, the efficacy of mizoribine has differed among various reports, depending on the dose given. There have yet to be any conclusive reports on the effects of mizoribine in SRNS in children.
Better results might be obtained if the doses of mizoribine increased in children with FRNS. When 5 mg/kg was used, no serious adverse effects were seen, therefore this dose may be safe and effective. An investigation of appropriate, effective and safe doses of mizoribine should be examined in the future. In patients with SRNS, large doses of mizoribine of more than 5 mg/kg might be effective, while the combined use of mizoribine and cyclosporin or methylprednisolone pulse therapy might also be even more effective. Further studies are called for to examine the use of large doses of mizoribine.
肾病综合征（NS）的治疗近来取得了显著进展。治疗的最终目的是让患者能够过上正常的、无疾病的生活，且无治疗带来的不良影响。然而，对于频繁复发型肾病综合征（FRNS）和激素抵抗型肾病综合征（SRNS）患儿，仍有待确立明确的治疗指南。频繁使用或大剂量使用类固醇可能会导致严重的不良反应。包括环磷酰胺和环孢素在内的免疫抑制药物已被用于诱导持久缓解，从而使患者避免进一步接触类固醇。然而，这些药物都有急性和慢性副作用。咪唑立宾是一种相对安全有效的免疫抑制剂。本报告讨论了咪唑立宾在儿童FRNS和SRNS中的作用。
回顾了关于咪唑立宾治疗NS的实验和临床研究论文。还补充了我们使用咪唑立宾的经验。
已有报道称咪唑立宾对FRNS患者是一种有效且安全的药物。然而，根据给药剂量不同，各报告中咪唑立宾的疗效有所差异。关于咪唑立宾对儿童SRNS的影响，尚未有任何结论性报告。
对于FRNS患儿，增加咪唑立宾剂量可能会取得更好的效果。使用5mg/kg时，未观察到严重不良反应，因此该剂量可能安全有效。未来应研究咪唑立宾合适、有效且安全的剂量。对于SRNS患者，超过5mg/kg的大剂量咪唑立宾可能有效，同时咪唑立宾与环孢素联合使用或甲泼尼龙冲击疗法可能会更有效。需要进一步研究来探讨大剂量咪唑立宾的使用。
